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Hurler Syndrome Back Surgery

Hurler syndrome, also known as MPS I, is a rare, genetic disease that prevents the body from breaking down sugars. The resulting buildup of molecular sugar chains can cause damage to many organs. Symptoms range from mild to severe and usually begin in childhood. Some of these symptoms can be alleviated through back surgery; however, the effects of Hurler syndrome can greatly increase risks during the operation.

  1. Reasons for Back Surgery

    • MPS I often affects the formation and growth of bones. Poorly formed vertebrae in the neck could lead to life-threatening injuries to the spinal cord. Fusion surgery connects the bones to keep them from slipping further out of place. Surgery may also be necessary to free trapped nerves or nerve roots caused by abnormal development. Without surgery, nerves could become compressed, leading to nerve damage, paralysis or death.

    Anesthesia Risks

    • Hurler syndrome can cause special problems for anesthesiologists during surgery. If abnormalities such as narrow nasal passages or enlarged tonsils, adenoids, lips and tongue exist, the patient's airway will be restricted. Because the relaxing nature of general anesthesia and its related medications also cause airway restriction, the patient is at high risk for having his airway compromised during surgery. Symptoms can also make it difficult to view the larynx. Extra tissue may encircle it and the instability of the spine may make it impossible to place the patient in the best position to see it.

    Other Risks

    • Due to thicker skin and stiff joints, obtaining an IV line in an emergency situation can be difficult. Deformed spines can restrict and compromise the lungs. The effects of Hurler syndrome can also cause problems for the heart, nerves, muscles, bones and spinal cord during surgery. Weakened bones make recovery difficult. More than one surgery is often required.

    Reducing Risks

    • Schedule the surgery at a major hospital with highly skilled and experienced staff. Choose an anesthesiologist who's had significant experience with Hurler syndrome patients. If it's determined that surgery could compromise the patient's airway, the anesthesiologist may recommend using a laryngeal mask airway instead of the endotracheal tube traditionally used. A sleep study can help assess the amount of airway restriction before surgery takes place. X-rays and lung function tests can also determine the specific risks for each patient.

    Prognosis

    • A case study cited in the Spine Journal documented successful back surgery on a 59-year-old Hurler syndrome patient in 2003. After surgery, the patient showed significant recovery. A larger study conducted in 2005 and reported by the British Scoliosis Society had mixed results. Dr. Colin E. Bruce, the orthopaedic surgeon who prepared the report, concludes that if the need for surgery is not life-threatening, surgery may be counterproductive.

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